CASE REPORT

Successful Pregnancy Outcome with Extra Hepatic Portal VenousObstruction: Three Case Series

Nilesh C. Mhaske1 • S. Madhva Prasad1 • Deepali Kharat1 • Michelle N. Fonseca1

Received: 7 November 2015 / Accepted: 7 May 2016 / Published online: 18 June 2016

� Federation of Obstetric & Gynecological Societies of India 2016

About the Author

Introduction

Pregnancy associated with portal hypertension is a high-risk

condition as both pregnancy and portal hypertension con-

tribute to worsening of hemodynamic status. The adaptive

changes worsen the portal hypertension and can result in

potentially life-threatening variceal bleeding and associated

complications. The liver function is much better preserved in

non-cirrhotic portal hypertension than in those associated

with cirrhosis. The exact incidence of non-cirrhotic portal

hypertension in pregnancy is not known [1]. The manage-

ment of such patients requires multidisciplinary approach,

preferably in a tertiary care institution. Three such success-

fully managed patients are discussed here.

Cases

Case 1

A 32-year-old primigravida, married since 12 years, pre-

sented at 28-week gestation with loose motions for 2 days

and swelling of feet since 2 weeks. Patient had undergone

treatment for infertility, leading to conception. Pallor was

present. Facial, vulval and bilateral pedal edema was pre-

sent. There were no signs of hepatocellular involvement.

Nilesh C. Mhaske, M.B.B.S., 3rd year postgraduate student; Madhva

Prasad S, M.S., Senior Resident; Deepali Kharat, M.S., Assistant

professor; Michelle N. Fonseca, M.S., Additional professor and Head

of the Unit at Department of Obstetrics and Gynecology, LTMGH

and LTMMC, Sion, Mumbai.

& Nilesh C. Mhaske

dr.nil12688@gmail.com

1 Department of Obstetrics and Gynecology, LTMGH and

LTMMC, Sion, Mumbai 400022, India

Nilesh Mhaske is a postgraduate student in LTMGH and has been taking keen interest in research related to Obstetrics and

Gynecology. His future interests include community obstetrics and ensuring implementation of standard practices in low-

resource settings.

The Journal of Obstetrics and Gynecology of India (November–December 2016) 66(S2):S694–S697

DOI 10.1007/s13224-016-0911-1

123

Obstetric examination was within normal limit. For pan-

cytopenia with severe hypo-proteinemia, 1 unit of blood

and 2 units of 20 % human albumin were transfused. Other

liver and kidney parameters were within normal limit.

Urine examination showed nephrotic range proteinuria and

gross hematuria. DCT/ICT was negative. Ultrasonography

showed splenomegaly (Fig. 1) with multiple dilated col-

laterals within the splenic hilum; chronic thrombosis of

portal and splenic vein; altered liver echo texture and

minimal ascites suggestive of EHPVO. Specialist gastro-

medicine and nephrology opinion was taken for further

management. Surveillance endoscopy showed no esopha-

geal or gastric varices. ANA, LA, aCLA were negative,

and ASO titer was normal. Compliment C3, C4 levels were

low, and hence, patient was started on Tab. deflazacort

30 mg once a day and followed up regularly in antenatal

outpatient department and a post-delivery renal biopsy was

planned. Patient developed pre-eclampsia at 30 weeks of

gestation requiring anti-hypertensives. Patient went into

spontaneous labor at gestational age of 34 weeks. Ten units

platelet transfused peri-delivery and a female child of

weight 1560 g delivered vaginally. Hematological, hepatic

and renal parameters dramatically improved around

2 weeks post-delivery, and hence, renal biopsy was

deferred. Patient was kept under observation, also because

baby was in NICU. Patient was discharged on day 26 of

delivery, with complete resolution of symptoms.

Case 2

A 28-year-old primigravida married since 1 year presented

at 30 weeks of gestation with deranged sugars. EHPVO

had been diagnosed at the age of 9 years and was on oral

propranolol 40 mg. She underwent upper intestinal

endoscopy multiple times, requiring sclerotherapy thrice.

At 18 weeks of gestation, two large esophageal varices

necessitated variceal ligation.

On general examination pallor was present. There was

no icterus, edema or signs of liver cell failure. On per

abdominal examination, massive splenomegaly was pre-

sent. Obstetric examination was within normal limit.

Investigations revealed pancytopenia and deranged blood

sugar levels, while liver and kidney function tests were

within normal limit. Ultrasonography revealed altered liver

echostructure with central intra-hepatic biliary radicle

dilatation, and portal vein was replaced by multiple dilated

collaterals in the peri-portal area (Fig. 2). Gastro-medicine

specialist reviewed and advised no specific intervention.

Subcutaneous insulin was required to control blood sugars.

Induction of labor was done at 39 weeks, and due to non-

progress of labor, LSCS was done, with peri-operative

platelets and FFP transfusion, and delivered a healthy male

child of 3.1 kg. Post-LSCS, she required 3 units of blood

transfusion. Patient developed subcutaneous wound

hematoma which resolved spontaneously and severe ascites

requiring 1 unit of 20 % human albumin transfusion.

Case 3

A 31-year-old primigravida, married since 1 year, was a

diagnosed case of EHPVO. Patient had one episode of

hematemesis 7 years back, requiring variceal ligation and

was on tab. propranolol 40 mg since then. Endoscopy at

20-week gestation was suggestive of esophageal varices

with portal hypertensive gastropathy (Figs. 3, 4) and was

advised variceal ligation. However, patient defaulted and

Fig. 1 Ultrasonography showing splenomegaly

Fig. 2 Colour Doppler showing narrowed portal vein with peri-portal

collaterals

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The Journal of Obstetrics and Gynecology of India (November–December 2016) 66(S2):S694–S697 Successful Pregnancy Outcome with Extra Hepatic…

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presented at 34 weeks of gestation. On general examina-

tion, pallor was present. There was no icterus, edema feet

or signs of liver cell failure. On per abdominal examina-

tion, massive splenomegaly was present and obstetric

examination was within normal limits. Blood investiga-

tions revealed pancytopenia, with normal liver and kidney

function tests. There was no associated proteinuria or

hematuria. Protein C, protein S, LA, aCLA, Factor V and

Factor VIII were negative. Ultrasonography revealed cau-

date lobe hypertrophy, portal vein obstruction and severe

splenomegaly with mild ascites (Fig. 5). With peri-opera-

tive platelet transfusion, elective LSCS was done at

39 weeks of gestation for breech presentation and deliv-

ered a male child of 3 kg. Post-operative period was

uneventful.

Discussion

EHPVO is a rare condition, especially in the geographical

location from where this study is being reported. The eti-

ological factors associated include home delivery and

umbilical sepsis [2]. EHPVO in pregnancy is rare, and very

few studies pertaining to it have been reported [3].

The mean maternal age in our study was 30 years,

whereas the mean age in a large case series was 24 years.

The disease was diagnosed in this pregnancy in only one of

the three. In a large case series, 55 % of the patients were

diagnosed during the current pregnancy. Though none of

our patients had variceal bleeding during pregnancy, a

45-patient series reported bleeding only in three patients

[4]. The occurrence of the same has been associated with a

higher incidence of abortion and perinatal death [5].

Two of our three patients went to term gestation. A term

birth is reported in 58 % of such pregnancies [4]. Two of

our patients required Cesarean section, both for obstetric

indications. Cesarean section was used in 53 % of deliv-

eries in a large case series [4]. In our study, the mean birth

weight was 2553 g. In a large study, it was around 2668 g

[5]. All three of our patients required peri-delivery platelet

transfusion. In a large study, 50 % of the patients required

platelet transfusion [6].

Overall, the outcome was favorable in all three patients.

An overall favorable outcome has been reported in all case

studies, and no maternal deaths have been reported [3–6].

Conclusion

Pregnancy with portal hypertension is not contraindicated

as was once believed. With better diagnostic and treatment

modalities, maternal and fetal outcomes are constantly

Figs. 3 and 4 Endoscopic picture showing esophageal varices and

portal hypertensive gastropathy

Fig. 5 Colour Doppler showing prominent hepatic artery

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696

improving. Physicians and obstetricians need to coordinate

well regarding individualization of treatment and be

observant regarding specific risks, which include hemor-

rhage (mainly due to variceal bleeding, aneurysmal rupture

and PPH) or hepatic failure. A multidisciplinary team

approach in a tertiary care set-up, which resulted in three

successfully managed patients, is reported here. Further

prospective studies are needed in this regard.

Compliance with Ethical Standards

Conflict of interest None.

Ethical Standards All authors declare that all applicable ethical

standards have been complied with.

Human and Animal Rights This study did not involve any use of

animals.

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